产品标准

浓缩液(1mg/1ml) 亲和纯化抗体

 

产品应用

WB=1:100-500 Elisa =1:200-1000 IP=1:20-100 IHC=1:100-500    （石蜡切片需做抗原修复）

 

产品介绍

    多囊肾（polycystic kidney disease）为遗传性疾病，是肾脏一种先天性异常。双侧肾脏披髓质均可累及，但在程度上可不同。在遗传方式上表现为常染色体显性和常染色体隐性遗传两种。
    囊内上皮细胞异常增殖是ADPKD的显著特特之一，处于一种成熟不完全或重发育状态，高度提示为细胞的发育成熟调控出现障碍，使细胞处于一种未成熟状态，从而显示强增殖性。表现为细胞转运密切相关的Na+-K+-ATP ase的亚单位组合，分布及活性表达的改变；细胞信号传导异常以及离子转运通道的变化。细胞外基质异常增生是ADPKD第三种显著特征。目前许多研究已证明：这些异常均有与细胞生长有关的活性因子的参与。但关键的异常环节和途径尚未明了。因基因缺陷而致的细胞生长改变和间质形成异常，是本病的重要发病机制之一。    Mutations in the polycystin 2 gene are thought to be involved in autosomal dominant polycystic kidney disease, a condition characterised by renal cyst formation. Polycystin 2 has multiple membrane spanning domains with C and N terminal cytoplasmic domains, and has been found to be localised to the plasma membrane, ER and Golgi in various cell types and in cilia. It is thought to be involved in calcium signaling and transport and has been implicated in the development and morphology of renal tubules. It has been found to be regulated by EGF signalling. Interaction with polycystin 1 via the C terminus of both proteins is thought to be important for its function.
    mol wt:106kDa
    Other Aliases:
    polycystic kidney disease 2; TRPP2; APKD2, C030034P18RIK, MGC138466, MGC138468, PC2, PKD2 (includes EG:5311), PKD4, POLYCISTIN-2, POLYCYSTIN 2, RGD1559992, TRPP2; Polycystic kidney disease 2 protein homolog; PC2